Regulation of B-cell lymphoma/leukemia 11A on fetal hemoglobin

Sun Shaohua

doi:10.13406/j.cnki.cyxb.002492

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Regulation of B-cell lymphoma/leukemia 11A on fetal hemoglobin

更新时间：2024-01-09

- Regulation of B-cell lymphoma/leukemia 11A on fetal hemoglobin
- Journal of Chongqing Medical University Vol. 46, Issue 10, Pages: 1281-1286(2021)
- 作者机构：
  
  昆明理工大学医学院,昆明,650500
- 作者简介：
- 基金信息：
- DOI：10.13406/j.cnki.cyxb.002492
  CLC： R394
- Published：2021，
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Sun Shaohua. Regulation of B-cell lymphoma/leukemia 11A on fetal hemoglobin. [J]. Journal of Chongqing Medical University 46(10):1281-1286(2021)
DOI：

Sun Shaohua. Regulation of B-cell lymphoma/leukemia 11A on fetal hemoglobin. [J]. Journal of Chongqing Medical University 46(10):1281-1286(2021) DOI： 10.13406/j.cnki.cyxb.002492.

摘要

β-地中海贫血（β-thalassemia，β-地贫）和镰状细胞贫血（sickle cell anemia，SCA）均属于β-珠蛋白异常导致的血红蛋白病，不同程度地流行于东南亚、南亚、北非及地中海区域。胎儿血红蛋白（fetal hemoglobin，HbF）水平升高可明显改善两者的临床症状。B细胞淋巴瘤因子11A（B cell lymphoma/leukemia 11A，BCL11A）是一种锌指结构转录因子，在胎儿到成人血红蛋白转换过程中发挥重要的负向调节作用。BCL11A下调会激活γ-珠蛋白提高HbF表达，使β-地贫和SCA临床症状得到缓解。本文主要论述BCL11A对γ-珠蛋白的调控机制、BCL11A与β-地贫和SCA的治疗，从而为β-珠蛋白病的研究提供理论依据。

Abstract

β-thalassemia and sickle cell anemia (SCA) are hemoglobin diseases caused by abnormal β-globin

occurring in South-east Asia

South Asia

North Africa and the Mediterranean region with different degrees. Elevated fetal hemoglobin (HbF) levels can significantly improve their clinical symptoms. B-cell lymphoma/leukemia 11A (BCL11A)

a zinc finger structure transcription factor

plays an important negative regulatory role in fetal-to-adult hemoglobin expression. Down-regulated BCL11A activates γ-globin

so as to increase fetal hemoglobin expression

alleviating clinical symptoms of β-thalassemia and SCA. This article focuses on the mechanism of BCL11A on γ-globin

and the treatment of β-thalassemia and SCA based on BCL11A

thereby providing a theoretical basis for the study of β-globin disease.

关键词

胎儿血红蛋白镰状细胞贫血β-地中海贫血γ-珠蛋白B细胞淋巴瘤因子11A

Keywords

fetal hemoglobinsickle cell anemiaβ-thalassemiaγ-globinB-cell lymphoma/leukemia 11A

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